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Abstract Details

Three cases of Creutzfeldt-Jakob Disease after Hurricane Maria in Puerto Rico: Is this a coincidence?
Infectious Disease
P12 - Poster Session 12 (12:00 PM-1:00 PM)
13-007

To report three cases of male patients presenting with rapidly progressive dementia the year after Hurricane Maria in Puerto Rico.

Prion diseases, a group of disorders caused by abnormally shaped proteins called prions, occur in sporadic, genetic and acquired forms. The classic clinical phenotype is a rapidly progressive dementia with behavioral abnormalities, ataxia, extrapyramidal features, myoclonus, and eventually, death. Very few cases of CJD have been described at Puerto Rico during the last years prior to Hurricane Maria. 
Case Reports

We present three case reports of male patients presenting with rapidly progressive dementia the year after Hurricane Maria within a four-month period.

Case 1: 67 y/o male presented with memory difficulty and behavioral changes.Upon examination, patient was oriented only in place and person. Brain MRI showed gyriform multifocal signal abnormality and restricted diffusion involving bilateral cerebral hemispheres and subtle abnormality in medial thalami. Lumbar puncture was performed and 14-3-3 protein was detected in the cerebrospinal fluid. Patient died six weeks after evaluation.

Case 2: 82 y/o male presented with progressive mental status deterioration, marked dysarthria and postural tremor. Brain MRI findings consistent with large areas of hyperintensities in the cortical region of both occipital, temporal and left parietal and frontal lobes. Lumbar puncture was performed but 14-3-3 protein was not detected in the cerebrospinal fluid. Patient died four months after evaluation.

Case 3: 77 y/o male presented with functional decline. Examination was pertinent for hyperreflexia bilaterally with sustained clonus. Brain MRI showed hyperintensities at cortex. Lumbar puncture was performed but 14-3-3 protein was not detected in the cerebrospinal fluid. Patient died two weeks after evaluation and autopsy was performed that showed abnormal prion protein by Western blot.

These three cases stress the importance of considering additional, though less common, potential etiologies in patients with rapidly progressive dementia.

Authors/Disclosures
Maria E. Garcia Ayala, MD
PRESENTER
No disclosure on file
Rhaisa Castrodad- Molina, MD Dr. Castrodad- Molina has nothing to disclose.
Ernesto Borrero-Quintana, MD, PhD No disclosure on file
David Blas-Boria, MD, FÂé¶¹´«Ã½Ó³»­ (University of Puerto Rico) Dr. Blas-Boria has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novocure.
Viviana I. Orozco, MD No disclosure on file
No disclosure on file